OBJECTIVE 1: To obtain basic information on the energy metabolism and related activities of mitochondrial and sarcoplasmic reticulum preparations isolated from dystrophic muscle. Myodystrophic and strain 129 dystrophic mice are employed as animal models of muscular dystrophy. The following approaches will be employed. (A). Further characterization of mitochondrial preparations: I. To identify the site(s) of impairment in the NADH CoQ and succinate CoQ regions of the respiratory chain by studying various partial reactions involved in these regions of the respiratory chain. II. To elucidate the mechanism of defective oxidative metabolism of fatty acids by studying various enzyme reactions linked to fatty acid oxidaton. III. To characterize the functional aspects of the mitochondrial inner membrane by studying cation transporting activities. (B). Further characterization of sarcoplasmic reticulum preparations: I. To identify the optimal conditions for ATP hydrolysis and Ca ions transport by a systematic investigation of the effect of various biochemical parameters. II. To analyze the protein compostion of the membrane by means of gel electrophoresis. Comparison of these parameters derived from dystrophic muscle with those of controls should provide information on the mechanism of muscle degeneration and contribute to our understanding of the regulation of cellular energy metabolism of both healthy and diseased muscle. OBJECTIVE 2: To elucidate the action of Reye's Syndrome serum on mitochondrial metabolism. A systematic investigation of the effect of Reye's Syndrome serum on various electron transfer and energy transfer reactions is planned. This should add knowledge to our understanding of this grave puzzling illness.